• Neuromyelitis optica spectrum disorders (NMOSD), including neuromyelitis optica (NMO), are autoimmune diseases characterized by acute inflammation of the optic nerve (optic neuritis, ON) and the spinal cord (myelitis). (wikipedia.org)
  • However, with the discovery of disease-causing antibodies, a broader spectrum of disease manifestations has been grouped with NMO into the diagnosis of NMOSD. (wikipedia.org)
  • Lesions may also affect the diencephalon, mostly in Aquaporin-4-Immunoglobulin-G (AQP4-IgG) NMOSD. (wikipedia.org)
  • NMOSD is caused by an autoimmune attack on the nervous system. (wikipedia.org)
  • citation needed] NMOSD is usually caused by autoantibodies targeting aquaporin 4 (AQP4), a channel protein in the cell membrane that allows water to pass through the membrane. (wikipedia.org)
  • Thus, NMOSD involving AQP4-IgG can be considered an astrocytopathy or autoimmune astrocytic channelopathy, since the astrocytes are semi-selectively destroyed. (wikipedia.org)
  • Neuro-ophthalmic manifestations frequently are encountered in persons with multiple sclerosis (MS) and other central nervous system (CNS) inflammatory disorders, including neuromyelitis optica spectrum disorders (NMOSD) and myelin oligodendrocyte glycoprotein antibody associated disease (MOGAD). (medscape.com)
  • We tested this approach in 12 patients, 8 with MS and 4 with NMOSD. (guthyjacksonfoundation.org)
  • Background The presence of antibodies to aquaporin-4 (AQP4) has been identified as a key characteristic of neuromyelitis optica spectrum disorder (NMOSD), an autoimmune inflammatory demyelinating central nervous system (CNS) disorder. (guthyjacksonfoundation.org)
  • Here we investigated whether the brain structural and functional connectivity alterations were involved in fatigue related to neuromyelitis optica spectrum disorder (NMOSD). (biomedcentral.com)
  • Neuromyelitis optica spectrum disorder (NMOSD) is an autoimmune disorder affecting the central nervous system and associated with the presence of aquaporin-4 immunoglobulin G antibodies (AQP4-IgG) [ 1 ]. (biomedcentral.com)
  • Neuromyelitis optica spectrum disorder (NMOSD) is one of the autoimmune demyelinating disorders, which is thought to be triggered by viral infection. (aku.edu)
  • keywords = "Aquaporin 4 (AQP-4), Neuromyelitis optica spectrum disorder (NMOSD), Severe acute respiratory syndrome (SARS). (aku.edu)
  • The topics are in the areas of diagnosis and management of MS, NMOSD, MOGAD and related disorders. (pactrims.org)
  • In this article we will explore the orphan drug market by examining neuromyelitis optica spectrum disorder (NMOSD), an autoimmune disease that primarily affects the optic nerves and spinal cord. (optum.com)
  • 4 Originally developed to treat an unrelated blood disease, Soliris was granted the new indication after it was found to reduce attacks or relapses in NMOSD patients. (optum.com)
  • On June 27, 2019, eculizumab, or Soliris, became the first FDA approved treatment for anti-aquaporin-4 (AQP4) antibody positive neuromyelitis optica spectrum disorder (NMOSD) in adults. (clevelandclinic.org)
  • Findings published in PNAS from a study including individuals with neuromyelitis optica spectrum disorder (NMOSD) suggest that germinal center activity may play a targetable and modifiable role in autoimmune disorders. (neurologylive.com)
  • All told, investigator Sarosh R. Irani, DPhil, FRCP, FEAN, Head, Oxford Autoimmune Neurology Group, University of Oxford, and colleagues, assessed samples of deep cervical lymph node (dCLN) aspirates (n = 36) and blood (n = 406) from 63 patients with NMOSD. (neurologylive.com)
  • Jennifer van Amerom (she/her) received her diagnosis of neuromyelitis optica spectrum disorder - aquaporin-4 positive (NMOSD-AQP4) in early 2010. (neuromyelitisnews.com)
  • 2 3 4 5 6 ] However, a more recent population-based survey conducted in urban Mangalore has shown a prevalence of 8.3/100,000 for MS and 2.6/100,000 for neuromyelitis optic spectrum diseases (NMOSD). (lww.com)
  • 7 ] NMOSD is likely to constitute approximately 20% of all demyelinating disorders in India. (lww.com)
  • Certain neurological symptoms of neuromyelitis optica spectrum disorder (NMOSD) occur at higher rates in patients who also have systemic lupus erythematosus (SLE), according to a recent review study. (neuromyelitisnews.com)
  • Features of NMOSD, including longitudinally extensive transverse myelitis - a type of spinal cord inflammation - and involvement of certain areas of the brain were seen at higher rates in patients with both autoimmune diseases than usually observed in NMOSD alone. (neuromyelitisnews.com)
  • Both autoimmune disorders, NMOSD and lupus are each characterized by the immune system wrongfully attacking the body's own tissues. (neuromyelitisnews.com)
  • NMOSD patients were positive for aquaporin-4 (AQP4) antibodies, the most common type of NMOSD-causing antibody. (neuromyelitisnews.com)
  • UPLIZNA is the first and only anti-CD19 B-cell-depleting humanized monoclonal antibody approved by the U.S. Food and Drug Administration (FDA) and European Commission (EC) for the treatment of adult patients with anti-aquaporin-4 (AQP4) antibody positive NMOSD. (minstercommunitypost.com)
  • NMOSD is a unifying term for neuromyelitis optica (NMO) and related syndromes. (minstercommunitypost.com)
  • UPLIZNA is indicated for the treatment of neuromyelitis optica spectrum disorder (NMOSD) in adult patients who are anti-aquaporin-4 (AQP4) antibody positive. (minstercommunitypost.com)
  • Detection of aquaporin-4 immunoglobulin G antibodies (AQP4-IgG), using a blood test that was developed by Mayo Clinic investigators, is highly specific for NMOSD and facilitates the diagnosis. (sciencedaily.com)
  • The overlapping clinical features of relapsing remitting multiple sclerosis (RRMS), aquaporin-4 (AQP4)-antibody (Ab) neuromyelitis optica spectrum disorder (NMOSD), and myelin oligodendrocyte glycoprotein (MOG)-Ab disease mean that detection of disease specific serum antibodies is the gold standard in diagnostics. (biomedcentral.com)
  • While some features of MS and NMOSD do overlap, these two conditions are now understood to be distinct disorders, Dr Cohen said. (medscape.com)
  • Interestingly, it seems that about 20% of patients who are seronegative for the aquaporin-4 protein and who have NMOSD features also have antibodies against myelin oligodendrocyte glycoproteins (MOGs). (medscape.com)
  • Whether we will end up considering this to be 'MOG-related NMOSD' or whether we will consider it a new disorder remains to be seen," he said. (medscape.com)
  • Tumefactive Demyelination in MOG Ab-Associated Disease, Multiple Sclerosis, and AQP-4-IgG-Positive Neuromyelitis Optica Spectrum Disorder. (mayo.edu)
  • The study, " Overlap syndrome of anti-aquaporin-4 positive neuromyelitis optica spectrum disorder and systemic lupus erythematosus: A systematic review of individual patient data ," was published in the journal Lupus . (neuromyelitisnews.com)
  • In more than 80% of cases, IgG autoantibodies against aquaporin-4 (anti-AQP4+) are the cause, and in 10-40% of the remaining cases, IgG antibodies against MOG are the cause. (wikipedia.org)
  • Progression to wheelchair dependence was faster among patients with paraneoplastic compared with nonparaneoplastic autoimmune ataxia and among those with positivity for neuronal nuclear and cytoplasmic antibodies (NNC Abs + ) only (vs positivity for plasma membrane protein Abs [PMP Abs + ] only). (jamanetwork.com)
  • Multiple Sclerosis (MS) is a CNS disorder that is characterized by both inflammatory and neurodegenerative mechanisms of brain and spinal cord injury. (medscape.com)
  • Neuromyelitis optica following thymectomy with severe spinal cord atrophy after frequent relapses for 30 years]. (guthyjacksonfoundation.org)
  • Keywords:Devic's syndrome;neuromyelitis optica;plasma exchange;apheresis;steroid-refractory;MRI Abstract Neuromyelitis optica (NMO) is a severe inflammatory demyelinating disease of the central nervous system with exacerbations involving the optic nerves, spinal cord, or both. (guthyjacksonfoundation.org)
  • Neuromyelitis optica spectrum disorder is a demyelinating disorder that predominantly affects the eyes and spinal cord but can affect other structures in the central nervous system (CNS) that contain aquaporin 4. (msdmanuals.com)
  • In neuromyelitis optica spectrum disorder, the immune system targets aquaporin 4, a protein that is present on astrocytes in the brain and particularly the spinal cord and optic nerves, or myelin oligodendrocyte glycoprotein (MOG), which is present on the myelin of oligodendrocytes in the same areas of the CNS and possibly other targets. (msdmanuals.com)
  • Neuromyelitis optica affects several (typically ≥ 3) contiguous spinal segments of the spinal cord, whereas MS typically affects a single segment. (msdmanuals.com)
  • In patients with neuromyelitis optica (NMO), binding of NMO-IgG antibody to astrocytes results in uncontrolled complement activation and destruction of myelin-producing cells, leading to severe damage to the central nervous system and predominantly impacting the spinal cord and optic nerve. (physicianeditorial.com)
  • Myelopathy can be the result of primary intrinsic disorders of the spinal cord or from secondary conditions, which result in extrinsic compression of the spinal cord. (bvsalud.org)
  • PACTRIMS is an annual event that aims to provide clinical and scientific updates in the care of patients with multiple sclerosis (MS), neuromyelitis optica and related conditions in the Asia-Pacific region. (pactrims.org)
  • MOGAD is typically associated with acute disseminated encephalomyelitis (ADEM), optic neuritis (ON), and transverse myelitis (TM) and is less commonly associated with cerebral cortical encephalitis, brainstem or cerebellar symptoms, and clinical presentations including the combination of several phenotypes and sometimes accompanies other autoantibodies such as anti- N -methy-D aspartate receptor (NMDAR) autoantibodies with symptoms of autoimmune encephalitis. (encyclopedia.pub)
  • Q. What did the clinical trials of eculizumab in neuromyelitis optica show? (clevelandclinic.org)
  • Data assessing samples of deep cervical lymph node aspirates and blood suggest that germinal center activity was associated with clinical relapses in individuals with neuromyelitis optica spectrum disorder. (neurologylive.com)
  • They may explain rituximab's clinical efficacy in several autoantibody-mediated diseases and highlight the potential value of direct [germinal center] measurements across autoimmune conditions," Irani et al wrote. (neurologylive.com)
  • Neurologic manifestations are among the features of systemic lupus erythematosus (SLE), a multisystem autoimmune connective tissue disorder with various clinical presentations. (medscape.com)
  • Moreover, other conditions along the suggested continuum include optic neuritis, transverse myelitis, and neuromyelitis optica - clinical entities that may occur as manifestations of either MS or ADEM. (medscape.com)
  • Abstract Neuromyelitis optica is an autoimmune disorder characterized by optic neuritis, transverse myelitis, and aquaporin-4 autoantibodies. (guthyjacksonfoundation.org)
  • 4 , 5 , 7 The estimated frequency of CNS involvement ranges from 10-60% in different reports, depending on the parameters studied (eg, patient selection, diagnostic criteria, etc). 4 ⇓ - 6 Patients with pSS can present with a wide range of focal or diffuse neurologic or psychiatric manifestations, including motor/sensory deficits, transverse myelitis, and cognitive impairment. (ajnr.org)
  • We searched PubMed and Medline for studies published during the last 10 years with the general term "optic neuritis" and specific terms like "typical optic neuritis," "atypical optic neuritis," "multiple sclerosis," "neuromyelitis optic neuritis," and "myelin oligodendrocyte glycoprotein antibody. (lww.com)
  • Optic neuritis is an inflammatory injury of the optic nerve that causes vision loss, which is common in MS and other CNS inflammatory disorders. (medscape.com)
  • Optic neuritis (ON) is a demyelinating inflammation of the optic nerve that often occurs in association with multiple sclerosis (MS) and, much less commonly, neuromyelitis optica (NMO). (medscape.com)
  • Diffuse and heterogeneous T2-hyperintense lesions in the splenium are characteristic of neuromyelitis optica. (guthyjacksonfoundation.org)
  • Brain Lesions May Serve as Diagnostic Criteria for Neuromyelitis Optica 2011;19(8):13. (guthyjacksonfoundation.org)
  • A majority of patients with neuromyelitis optica also experience disease-specific brain lesions that could help distinguish neuromyelitis optica from multiple sclerosis. (guthyjacksonfoundation.org)
  • We excluded patients who had had an acute attack fewer than 3 months before the study, who were seronegative for AQP4-IgG based on flow cytometry of serum [ 12 ], who had psychiatric or other neurological disorders, who had visible brain lesions on conventional MRI, or for whom MRI was contraindicated. (biomedcentral.com)
  • On MRI, cerebral white matter lesions are uncommon in neuromyelitis optica, unlike in MS. (msdmanuals.com)
  • 4 ⇓ - 6 The current data from MRI studies support an increased frequency of high signal intensity lesions in the periventricular and/or subcortical WM on FLAIR and T2-weighted imaging, observed mainly in patients with pSS and evidence of CNS disease. (ajnr.org)
  • Sjögren syndrome is a chronic systemic autoimmune disease that can be classified as primary Sjögren syndrome (pSS) when presenting in isolation or secondary when related to another connective tissue disease. (ajnr.org)
  • In more than 80% of cases, NMO is caused by immunoglobulin G autoantibodies to aquaporin 4 (anti-AQP4), the most abundant water channel protein in the central nervous system. (wikipedia.org)
  • In fact, MOG was initially identified as an immunodominant target for demyelinating autoantibodies in a guinea pig model of experimental autoimmune encephalomyelitis (EAE) [ 2 ] [ 3 ] . (encyclopedia.pub)
  • Over the years, extensive studies conducted in patients with MS have investigated the presence of anti-MOG autoantibodies using Western blotting and enzyme-linked immunosorbent assays targeting recombinant mouse MOG, without clear relation and specificity with MS [ 4 ] . (encyclopedia.pub)
  • Background Autoantibodies to glial, myelin and neuronal antigens have been reported in a range of central demyelination syndromes and autoimmune encephalopathies in children, but there has not been a systematic evaluation across the range of central nervous system (CNS) autoantibodies in childhood-acquired demyelinating syndromes (ADS). (bmj.com)
  • Once triggered, SLE's autoimmune reaction affects many sites through multiple mechanisms such as deposition of immune complexes, effects of cytokines and other chemical neuromodulators, direct attack by autoantibodies or activated leukocytes, and others. (medscape.com)
  • Rarely, NMO may occur in the context of other autoimmune diseases (e.g. connective tissue disorders, paraneoplastic syndromes) or infectious diseases. (wikipedia.org)
  • Other manifestations of immunosuppression, such as the development of autoimmune diseases or malignancies, may also occur with some of these therapies. (medilib.ir)
  • Overview discussions of the use of these agents in rheumatic disorders and oncologic diseases are found separately. (medilib.ir)
  • Most patients were female (91.3%), consistent with the known female predominance in both autoimmune diseases. (neuromyelitisnews.com)
  • Association Between the Single Nucleotide Polymorphism and the Level of Aquaporin-4 Protein Expression in Han and Minority Chinese with Inflammatory Demyelinating Diseases of the Central Nervous System. (cdc.gov)
  • The anterior optic pathway is one of the preferential sites of involvement in CNS inflammatory demyelinating diseases, such as multiple sclerosis and neuromyelitis optica, with optic neuritis being a common presenting symptom. (ox.ac.uk)
  • Astrocytes and oligodendrocytes are damaged by autoimmune-mediated inflammation as well as demyelination. (msdmanuals.com)
  • In neuromyelitis optica spectrum disorder, cases with a history of optic neuritis had extensive demyelination and lost aquaporin-4 reactivity. (ox.ac.uk)
  • Because of these results, a subsequent randomized, double-blinded, placebo-controlled phase III time-to-event trial over 4 years, Prevention of Relapses in Neuromyelitis Optica (PREVENT), was performed. (clevelandclinic.org)
  • His research focuses on what provokes relapses and remissions in multiple sclerosis (MS), and on the quest for antigen specific therapy in autoimmune disease. (neuroimmune.org)
  • Such fatigue has also been reported in a high percentage of patients with multiple sclerosis (MS), another autoimmune disease affecting the central nervous system. (biomedcentral.com)
  • Exclusion criteria were a history of major metabolic, neurologic, or psychiatric disorder and high risk for cardiovascular disease. (ajnr.org)
  • 4 ⇓ - 6 Although involvement of the peripheral nervous system is a well-documented feature of the disease, the prevalence, the type, and the underlying mechanism of CNS involvement remain unclear. (ajnr.org)
  • 4,6 Loss of vision, paralysis, loss of sensation, bladder and bowel dysfunction, nerve pain and respiratory failure can all be manifestations of the disease. (minstercommunitypost.com)
  • In this article, the disease-related and treatment-related neurological complications of renal disorders will be reviewed. (neurologyindia.com)
  • Our findings suggest that chronic inflammation is frequent and leads to neurodegeneration in multiple sclerosis and neuromyelitis optica, regardless of disease stage. (ox.ac.uk)
  • 4-6 Patients with NMO have a life-long exposure to the uncontrolled terminal complement activation due to chronic autoimmune attack, and most patients experience an unpredictable, relapsing course of disease with cumulative disability, as each attack adds to the neurologic disability. (physicianeditorial.com)
  • Many patients with neurological disorders experience chronic fatigue, but the neural mechanisms involved are unclear. (biomedcentral.com)
  • Actually, in neurological disorders, defects in certain brain regions may be at fault, such as in pathways interconnecting the basal ganglia, thalamus, and higher cortical centers, and pathways involved in hypothalamic-pituitary-diencephalic syndrome [ 11 ]. (biomedcentral.com)
  • The disorder manifests as an acute-onset encephalopathy associated with polyfocal neurologic deficits and is typically self-limiting. (medscape.com)
  • Serum anti-neuromyelitis optica (NMO)/aquaporin-4 (AQP4) antibody was positive and was confirmed by repeat serology (cell-based assay, Mayo Clinic). (neurology.org)
  • Affected individuals may experience problems with how they see the world (afferent visual pathway symptoms) and/or how smoothly and synchronously their eyes move together (efferent visual pathway disorders). (medscape.com)
  • Because patients with MS and other CNS inflammatory disorders who have visual symptoms often seek ophthalmic attention, eye care experts play a vital role in the localization and diagnosis of these conditions. (medscape.com)
  • Symptoms of neuromyelitis optica spectrum disorder include visual loss, muscle spasms, paraparesis or quadriparesis, and incontinence. (msdmanuals.com)
  • It can cause a spectrum of symptoms, including visual loss, paralysis and episodes of persistent hiccups, nausea and vomiting. (sciencedaily.com)
  • Patients with visual disturbances in the setting of suspected or established multiple sclerosis (MS) or other CNS inflammatory disorder should be cautioned to avoid activities that present a safety risk to themselves or others. (medscape.com)
  • Immune tolerance in multiple sclerosis and neuromyelitis optica with peptide-loaded tolerogenic dendritic cells in a phase 1b trial. (guthyjacksonfoundation.org)
  • There are adaptive T-cell and antibody autoimmune responses to myelin-derived peptides in multiple sclerosis (MS) and to aquaporin-4 (AQP4) in neuromyelitis optica spectrum disorders (NMOSDs). (guthyjacksonfoundation.org)
  • Multiple sclerosis and related disorders 2018 Mar 21 97-102. (cdc.gov)
  • The functional status and medical fragility of the patient - Studies have shown that hospitalized patients, those with poor functional status or comorbid conditions, and older adults are more likely to develop immune complications when treated with glucocorticoids, and this is likely to be the case for other immunosuppressant drugs [ 4 ]. (medilib.ir)
  • Eculizumab was first studied in 14 AQP4 antibody positive neuromyelitis optica patients in an open-label trial. (clevelandclinic.org)
  • Headache, nausea, and dizziness were the most common side effects reported in the open label trial of eculizumab in APQ4 antibody positive neuromyelitis optica patients. (clevelandclinic.org)
  • Preliminary study on the association of AQP4 promoter polymorphism with anti-aquaporin-4 antibody positivity in southern Han Chinese patients with idiopathic demyelinating disorders of central nervous system. (cdc.gov)
  • Autoimmune and immunogenetic profile of patients with optic neuritis in a population-based cohort. (cdc.gov)
  • CHESHIRE, Conn. November 25, 2014 -(BUSINESS WIRE)-Alexion Pharmaceuticals, Inc. (NASDAQ:ALXN) today announced that Soliris ® (eculizumab) has been granted orphan drug designation (ODD) by Japan's Ministry of Health, Labour and Welfare (MHLW) for the treatment of patients with neuromyelitis optica (NMO), a life-threatening, ultra-rare neurologic disorder. (physicianeditorial.com)
  • Soliris is a first-in-class terminal complement inhibitor and is currently approved in the United States, European Union, Japan and other countries for the treatment of patients with paroxysmal nocturnal hemoglobinuria (PNH) and atypical hemolytic uremic syndrome (aHUS), two debilitating, ultra-rare and life-threatening disorders caused by chronic uncontrolled complement activation. (physicianeditorial.com)
  • This patient is presented to draw attention to a rare neurological disorder which should be included in the differential diagnosis of nausea and vomiting with some key neurological complaints, even in the absence of physical findings. (hindawi.com)
  • As part of her ongoing research program, Dr. Severance focuses on the major gateway of the immune system, the gastrointestinal mucosa, where inflammation, food hypersensitivities, barrier defects and immune dysregulation can cause downstream brain dysfunction in people with psychiatric disorders. (neuroimmune.org)
  • The use of biologics to modulate the immune system has become a major therapeutic approach in the setting of inflammatory disorders. (medilib.ir)
  • Certain medications (e.g., phenytoin, hydralazine, procainamide, and isoniazid) may produce drug-induced lupus, but this disorder differs from classic SLE in its autoantibody profile (e.g., antihistone antibody positive) and in sparing the kidneys and CNS. (medscape.com)
  • Psychiatric researchers are looking beyond the central nervous system (CNS) for novel ways that the body's peripheral cellular and molecular pathways might be harnessed into effective treatments of brain disorders. (neuroimmune.org)
  • A prospective case-control study comparing optical coherence tomography characteristics in neuromyelitis optica spectrum disorder- optic neuritis and idiopathic optic neuritis. (cdc.gov)